progressive facial hemiatrophy

Cerebral
microhemorrhage • Parry-Romberg syndrome with intracranial manifestations - Ganzer Fall bei Radiopaedia
Saad, A. Parry-Romberg syndrome with intracranial manifestations. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-50949 • CC by-nc-sa 3.0 (modified)
A 3D
reconstruction of the CT scan of a 17 year old girl affected with Parry Romberg syndrome. It shows shrinkage of the subcutaneous soft tissue, fat tissue and muscle, with no apparent bone involvement.
Wikipedia • CC BY-SA 3.0
A CT scan of
a 17 year old girl with Parry Romberg syndrome, showing shrinkage and atrophy of the underlaying subcutaneous tissue and underlaying muscle, with no apparent involvement of the bone structure.
Wikipedia • CC BY-SA 3.0
A 3D
reconstruction of the CT scan of a 17 year old girl affected with Parry Romberg syndrome. Bone reconstruction.
Wikipedia • CC BY-SA 3.0
A 17 year old
girl with Parry Romberg syndrome.
Wikipedia • CC BY-SA 3.0
Imaging
spectrum of abnormal subcutaneous and visceral fat distribution. A 29-year-old man with Parry-Romberg syndrome. a Axial CT images show asymmetrically decreased subcutaneous fat on the left side (arrows). b Hypoplasty of the left orbit is also evident. c Volume-rendered three-dimensional reformation image of facial bones highlights bony asymmetry of the face. There is a perceivable asymmetry of the maxillary bone as well. d Coronal FLAIR image shows subcortical high signal intensity area (arrowhead)
insightsimaging.springeropen.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy (PFH), is a rare progressive craniofacial disorder (phakomatosis).

It is classically characterized by a slowly progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy). This involves the skin, underlying soft tissues, cartilaginous structures and bone.

Epidemiology

There may be slightly increased prevalence in females. It is thought to be of sporadic onset.

Clinical presentation

It typically presents in childhood. The overlying craniofacial skin in the affected region may be hyperpigmented.

Associations

The condition is often accompanied by:

Other possible associations include:

Radiographic features

CT/MRI brain
  • may show atrophy on one side of the face +/- scalp
  • high (T2/PD/FLAIR) signal of white matter within the ipsilateral and less commonly contralateral brain parenchyma has also been reported on MRI
  • ipsilateral and less often contralateral leptomeningeal enhancement
  • ipsilateral parenchymal atrophy
  • ipsilateral cerebral microhemorrhages
  • ipsilateral parenchymal calcification
  • intracranial aneurysms

History and etymology

Caleb Hillier Parry was a British physician (1755-1822), and Moritz Heinrich Romberg was a German neurologist (1795-1873).

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu progressive faziale Hemiatrophie:
Phakomatosen
 
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Rasmussen-Enzephalitis
 
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